Congenital cholesteatoma of the middle ear in children.

Congenital cholesteatoma, once considered to be a rarity, has shown an exponential increase in its incidence as evidenced by numerous clinical studies over the past decade. No explanation has been offered for this phenomenon. A review of the literature indicates that the definition of "congenital cholesteatoma" has strayed from the original one formulated by Derlacki and Clemis, which included a cholesteatoma medial to an intact tympanic membrane, without prior history of aural infections. Allowing patients with recurrent otitis media into studies may have contributed to a "false positive" rise in the incidence of this condition. This article reviews 19 cases of cholesteatomas behind intact tympanic membranes, treated at the Montreal Children's Hospital over a 10-year period. They represent 22% of the entire cholesteatoma population. There appears to be two groups of patients, each with a distinct clinical presentation that correlates well with the surgical outcome. Whether this implies different pathogenetic mechanisms remains unclear.